Miscellaneous Diseases of the Small Intestine

Levin, Marc S.; Gyawali, C. Prakash

doi:10.1002/9781444303254.ch55

Cited by 2 publications

(1 citation statement)

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“…A high-protein, low-fat diet, supplemented with medium-chain triglycerides is the simplest, most effective, and most widely prescribed treatment with the fewest side effects [18]. When dietary treatment is ineffective, and IL might be associated with autoimmune diseases, aggressive immunosuppressive regimens, including corticosteroids, cyclosporine, azathioprine, or cyclophosphamide, have been successfully used [19]. In this study, corticosteroids achieved complete or partial responses in 5 of 5 (100%) patients with the non-white villi type, including 2 cases that were secondary to systemic lupus erythematosus and living donor liver transplantation for congenital biliary atresia, respectively.…”

Section: Discussionmentioning

confidence: 99%

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Ohmiya

Nakamura²,

Yamamura³

et al. 2014

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Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. Results: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal E₁-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response. i 2014 S. Karger AG, Basel

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Section: Discussionmentioning

confidence: 99%